Rib myelolipoma: a case report.

BACKGROUND: Myelolipoma is an uncommon benign tumor composed of mature adipose tissue and hematopoietic elements. These tumors generally affect the adrenal glands, with anomalous presentations being rare and with few cases described in the literature. Most myelolipomas are asymptomatic and discovered incidentally, either through imaging tests or at autopsies. However, depending on the location and size of the lesion, myelolipomas can cause symptoms of mass effect. This article aims to report a very rare presentation of a symptomatic primary myelolipoma affecting the ribs. CASE PRESENTATION: A 21-year-old white female patient presented with a complaint of burning chest pain over 3 months, with gradual worsening in intensity, accompanied by a progressively growing bulge in the right thoracic wall. The patient underwent thoracotomy of the fifth and sixth ribs with complete excision of the lesion with a safety margin. Thoracic wall reconstruction was performed using a polypropylene mesh. The patient had a good postoperative course and was discharged on postoperative day 3. Histopathological examination revealed a histological image consistent with myelolipoma. CONCLUSIONS: This report underscores the importance of considering a myelolipoma diagnosis for tumor masses in the ribs.

Assessment of injuries patterns produced by a 9mm P.A.K "rubber ball" blank firing weapon: porcine model.

The use of less lethal weapons aims to mitigate civilian casualties caused by firearm use. However, due to numerous cases in which these weapons caused serious injuries, even lethal injuries, both legislation and the forensic field are interested in characterizing and regulating them better. In the forensic field, there is a lack of strong research about injury patterns of these weapons which makes it difficult to identify the type of weapon employed. In this study, the main objective was to characterize the injury pattern produced by the impact of the 9 mm P.A.K. projectile. A porcine model was used. Four different distances were studied: firm contact, 10 cm, 60 cm and 110 cm, using 3 of the more representative anatomical sites: the head, the hind leg and the ribs. The average measurement of the entrance orifice varied according to the anatomical site, being 6.67 mm wide and 6.25 mm long in the thorax, 7.3 mm wide and 8.8 mm long in the hind legs, and 7.62 mm wide and 7.54 mm long in the head. The variation in width and length measurements was not found to be directly related to the shot distance. The gunshot residues had similar characteristics to those of conventional lead projectiles, however there was more unburned powder deposit near the wounds, with a less dense soot and more dense powder tattoo. Depth varied widely regardless of tissue and firing distance, although loss of penetrating power and injury is observed as one moves away from the target.

Tumor de Células Gigantes em porção anterolateral de costela / Giant Cell Tumor in anterolateral rib portion

RESUMO: O Tumor de Células Gigantes é uma neoplasia osteolítica benigna, porém agressiva, ricamente vascularizada e com numerosas células gigantes com atividade osteoclástica. Ocorre mais frequentemente nas epífises de ossos longos e raramente acomete as costelas (cerca de 1% dos casos). A apresentação deste tumor em porção anterolateral de arco costal é extremamente rara em comparação com a região posterior. O presente relato descreve o caso de uma paciente que apresentava uma tumoração dolorosa em parede torácica anterolateral esquerda, de crescimento insidioso. Exames de imagem revelaram uma lesão insuflativa em 10º arco costal esquerdo (4,2 x 3,5 cm), com áreas de calcificação interior. A paciente foi submetida a tratamento cirúrgico, com ressecção ampla do tumor, englobando 9º, 10º e 11º arcos costais e uma porção do diafragma. O diagnóstico definitivo de tumor de células gigantes se deu pela imunohistoquímica da peça cirúrgica. Este relato permite fornecer bases para o estudo das possibilidades terapêuticas desta patologia em localizações atípicas, reforçando que a exérese em bloco com margens amplas destes tumores promove uma menor chance de recidiva local. (AU)

ABSTRACT: Giant Cell Tumor is a benign but aggressive osteolytic neoplasm, richly vascularized and with numerous giant cells with osteoclastic activity. It often occurs in long bone epiphyses and rarely affects the ribs (about 1% of cases). The presentation of this tumor in the anterolateral portion of the costal arch is extremely rare compared to the posterior region. The present report describes the case of a patient who had a painful tumor on the left anterolateral chest wall, of insidious growth. Imaging exams revealed an insufflating lesion in the 10th left costal arch (4.2 x 3.5 cm), with areas of interior calcification. The patient underwent surgical treatment, with wide resection of the tumor, comprising 9th, 10th and 11th ribs and a portion of the diaphragm. The definitive diagnosis of giant cell tumor was made by immunohistochemistry of the surgical specimen. This report provides a basis for studying the therapeutic possibilities of this pathology in atypical locations, reinforcing that the block excision with wide margins of these tumors promotes a lower chance of local recurrence. (AU)

Plasmocitoma solitario de costilla como presentación inicial de mieloma múltiple: reporte de un caso / Lone rib plasmacytoma as a presentation of initial multiple myeloma: a case report

Resumen Introducción: El 50% de los tumores de pared torácica son malignos, dentro de los que destaca el plasmocitoma de costilla. Objetivo: Presentar un caso clínico que debutó inicialmente como un plasmocitoma de costilla, y que terminó presentándose como mieloma múltiple. Materiales y Método: Registro clínico de un paciente sometido a resección de tumor de parrilla costal. Resultados: Paciente masculino de 58 años, con un año de dolor costal, asociado a aumento de volumen a nivel de la octava costilla derecha en línea media axilar, indurada. TC de tórax que demuestra imagen sugerente de plasmocitoma de 79 × 44 mm. Se realiza resección quirúrgica, con instalación de malla de prolene en el defecto. Biopsia diferida con compromiso neoplásico por lesión monoclonal de células plasmáticas. Se complementa estudio con biopsia de médula ósea confirmando mieloma múltiple. Se inicia tratamiento con quimioterapia adyuvante. Conclusiones: El plasmocitoma óseo solitario es una entidad de baja frecuencia, que se asocia a la presencia de mieloma múltiple. Es por esto que al momento de la sospecha se hace necesario descartar su presencia, con el fin de mejorar el pronóstico del paciente.

Introduction: Up to 50% of chest wall tumors are malignant; among which rib plasmocytoma stand out. Aim: Showcase a clinical case that debuted as a rib plasmacytoma, and that ended up presenting as Multiple Myeloma. Materials and Method: Records of a patient with resection of chest wall tumor. Results: Male patient of 58 years, with one year of costal pain, associated with an indurated increase in volume at the level of the eighth right rib in the mid-axillary line. Chest CT scan demonstrated a suggestive image of plasmacytoma of 79 × 44 mm. Surgical resection was performed, with prolene mesh installation in the defect. Biopsy showed neoplastic compromise due to monoclonal lesion of plasma cells. Study is complemented with bone marrow biopsy confirming multiple myeloma. The patient was treated with adjuvant chemotherapy. Conclusions: Solitary bone plasmacytoma is a low frequency entity, which is associated with the presence of multiple myeloma. At the moment of suspicion, it is necessary to rule out their presence, in order to improve the patient's prognosis.

Short stature, unusual face, delta phalanx, and abnormal vertebrae and ribs in a girl born to half-siblings.

Delta phalanx is a rare abnormality typically associated with additional features. We describe a patient with a phenotype resembling Catel-Manzke syndrome, but with delta phalanx and abnormal vertebrae and ribs. The patient was the only child of half siblings born with a marked prenatal growth deficiency. At 10 years of age, she had a short stature, long face, long and tubular nose with small alae nasi, high palate, short and broad thorax, and short index fingers with radial deviation. There were hyperpigmentations following Blaschko's lines. Radiology showed a proximal delta phalanx in the index finger of hands, abnormal vertebrae, and fused and small ribs. GTG-Banding karyotype and microarray analysis yielded normal results. Exome sequencing identified 25 genes that harbored homozygous variants, but none of these is assumed to be a good candidate to explain (part of) the phenotype. The here described patient may have a new condition, possibly following an autosomal recessive pattern of inheritance, although due to the high degree of consanguinity a compound etiology of the phenotype by variants in various genes may be present as well.

Osteosarcoma telangiectásico en un lactante / Telangiectatic osteosarcoma in an infant

Resumen: Introducción: El osteosarcoma es uno de los tipos de cáncer más común en la etapa escolar y en la adolescencia, y es el más común de los tumores óseos malignos en este grupo de edad. Frecuentemente, el osteosarcoma es hallado en los huesos largos de las extremidades. Existen muy pocos casos descritos en menores de 5 años. Hasta donde se sabe, no hay ningún caso reportado en lactantes. La variedad telangiectásica es poco común y no existen casos reportados antes de la edad preescolar. Caso clínico: Se presenta el caso clínico de una paciente lactante de 10 meses de edad que presentó un tumor lítico en la novena costilla derecha, que posterior a la resección fue clasificado como osteosarcoma telangiectásico. Conclusiones: El osteosarcoma telangiectásico en la edad pediátrica es poco común. A la fecha, la paciente ha presentado buena respuesta al tratamiento, aunque se desconoce el pronóstico y la supervivencia de este padecimiento.

Abstract: Background: Osteosarcoma is one of the most common types of cancer in childhood and adolescence and it is the most common malignant bone tumor in this group of age. Osteosarcoma is frequently found in long bones of the extremities. There are very few cases described in children under 5 years of age, and according to this review, none in infants. The telangiectatic variant is uncommon, and there are no reported cases before preschool age. Case report: A 10-month-old female infant with a lithic tumor of the ninth right rib, which was classified after resection as a telangiectatic osteosarcoma, is presented. Conclusions: Telangiectatic osteosarcoma in the pediatric age is very uncommon. To date, the patient has presented good response to treatment, although the prognosis and survival of this condition is unknown.

[Telangiectatic osteosarcoma in an infant]. / Osteosarcoma telangiectásico en un lactante.

BACKGROUND: Osteosarcoma is one of the most common types of cancer in childhood and adolescence and it is the most common malignant bone tumor in this group of age. Osteosarcoma is frequently found in long bones of the extremities. There are very few cases described in children under 5 years of age, and according to this review, none in infants. The telangiectatic variant is uncommon, and there are no reported cases before preschool age. CASE REPORT: A 10-month-old female infant with a lithic tumor of the ninth right rib, which was classified after resection as a telangiectatic osteosarcoma, is presented. CONCLUSIONS: Telangiectatic osteosarcoma in the pediatric age is very uncommon. To date, the patient has presented good response to treatment, although the prognosis and survival of this condition is unknown.

Condrossarcoma em gradil costal de cão: relato de caso / Chondrosarcoma in grating costal in a dog a case report / Condrosarcoma en costal rejilla en un perro - un informe del caso

O condrossarcoma é uma neoplasia maligna, de origem mesenquimal, cujas células neoplásicas produzem uma matriz cartilaginosa, não osteoide ou diretamente óssea. O diagnóstico pode ser auxiliado por meio de métodos imaginológicos, porém apenas a histopatologia é definitivo. O presente relato descreve o caso de um cão sem padrão racial definido, com sete anos de idade, o qual apresentou um aumento de volume com cerca de 6,0 x 5,0 x 4,0 cm na região do hemitórax esquerdo, o qual segundo o proprietário estava presente há dois meses. Após a histopatologia, foi realizado o diagnóstico de condrossarcoma, porém o proprietário não procedeu com o tratamento instituído e o animal retornou a clínica 11 meses após o primeiro incidente em sofrimento irreversível. Objetiva-se descrever os aspectos macro e microscópico da respectiva neoplasia.(AU)

Chondrosarcoma is a malignant tumor of mesenchymal origin, which the neoplastic cells produce a cartilage matrix, no osteoid or bone directly. Diagnosis can be aided by imaginological methods, but only histopathology is definitive. This report describes the case of a mongrel dog, seven years old, with a volume increase of about 6,0 x 5,0 x 4,0 cm in the left hemithorax, which according to the owner there was two months ago. Histopathology after the diagnosis of chondrosarcoma was made and treatment instituted, but as the owner lost the thread it was not performed and the animal returned to clinical 11 months after the first incident irreversible suffering. Objective is to describe the macro and microscopic aspects of the neoplasm.(AU)

El condrosarcoma es un tumor maligno de origen mesenquimal, cuyas células neoplásicas producir una matriz de cartílago, no osteoide o hueso directamente. El diagnóstico puede ser assistido por métodos de imagen, pero sólo la histología esdefinitiva. Este informe describe el caso de un perro sin raza definida, de siete anos, con un aumento de volumen de alrededor de 6,0 x 5,0 x 4,0 cm en el hemitórax izquierdo, que según el propietario estaba allí hace dos meses. Después se realizó el diagnóstico histopatológico de condrosarcoma y el tratamiento instituido, pero como el propietario pierde el hilo no se hizo y el animal volvió a clínicas 11 meses después del primer incidente en el sufrimiento irreversible. Su objetivo es describir los aspectos macroscópicos y microscópicos de su neoplasia.(AU)

Condrossarcoma em gradil costal de cão: relato de caso / Chondrosarcoma in grating costal in a dog – a case report / Condrosarcoma en costal rejilla en un perro - un informe del caso

O condrossarcoma é uma neoplasia maligna, de origem mesenquimal, cujas células neoplásicas produzem uma matriz cartilaginosa, não osteoide ou diretamente óssea. O diagnóstico pode ser auxiliado por meio de métodos imaginológicos, porém apenas a histopatologia é definitivo. O presente relato descreve o caso de um cão sem padrão racial definido, com sete anos de idade, o qual apresentou um aumento de volume com cerca de 6,0 x 5,0 x 4,0 cm na região do hemitórax esquerdo, o qual segundo o proprietário estava presente há dois meses. Após a histopatologia, foi realizado o diagnóstico de condrossarcoma, porém o proprietário não procedeu com o tratamento instituído e o animal retornou a clínica 11 meses após o primeiro incidente em sofrimento irreversível. Objetiva-se descrever os aspectos macro e microscópico da respectiva neoplasia.

Chondrosarcoma is a malignant tumor of mesenchymal origin, which the neoplastic cells produce a cartilage matrix, no osteoid or bone directly. Diagnosis can be aided by imaginological methods, but only histopathology is definitive. This report describes the case of a mongrel dog, seven years old, with a volume increase of about 6,0 x 5,0 x 4,0 cm in the left hemithorax, which according to the owner there was two months ago. Histopathology after the diagnosis of chondrosarcoma was made and treatment instituted, but as the owner lost the thread it was not performed and the animal returned to clinical 11 months after the first incident irreversible suffering. Objective is to describe the macro and microscopic aspects of the neoplasm.

El condrosarcoma es un tumor maligno de origen mesenquimal, cuyas células neoplásicas producir una matriz de cartílago, no osteoide o hueso directamente. El diagnóstico puede ser assistido por métodos de imagen, pero sólo la histología esdefinitiva. Este informe describe el caso de un perro sin raza definida, de siete anos, con un aumento de volumen de alrededor de 6,0 x 5,0 x 4,0 cm en el hemitórax izquierdo, que según el propietario estaba allí hace dos meses. Después se realizó el diagnóstico histopatológico de condrosarcoma y el tratamiento instituido, pero como el propietario pierde el hilo no se hizo y el animal volvió a clínicas 11 meses después del primer incidente en el sufrimiento irreversible. Su objetivo es describir los aspectos macroscópicos y microscópicos de su neoplasia.

Expanding the phenotype of spondylospinal thoracic dysostosis (the Turkel-Chen-Johnson syndrome).

Spondylospinal thoracic dysostosis can be considered a type of spondylocostal dysostosis because of the occurrence of vertebral defects (hemivertebrae and vertebral body fusion) and thoracic anomalies (short thorax and pulmonary hypoplasia). This syndrome was described by Johnson et al. (1997) in two siblings with dwarfism, short thorax, curved spine, fusion of the vertebrae and spinal process, multiple pterygium, and arthrogryposis. We describe the case of a 16-year-old Mexican girl with the longest survival recorded (the previous oldest patient was 7 years old) and analyze the natural history and describe some new features of this rare entity.

Breast asymmetry pattern in women with idiopathic scoliosis.

Breast asymmetry is frequent in women with idiopathic scoliosis. To understand the pattern of breast asymmetry in these women a clinical study was performed in which 54 female patients with idiopathic scoliosis were evaluated. The information recorded for each patient included: age, weight, height, scoliosis type, Cobb angle, breast measurements, and presence of rib cage asymmetry. Breast volume was calculated using anatomic measurements (anthropomorphic method). The mean age of the group was 25 +/- 7 years. A right convex thoracic curve occurred in 85%, with a mean Cobb angle of 32 +/- 15 degrees. Our study indicated that women with idiopathic scoliosis consistently presented breast asymmetry that followed a predictable pattern. The breast on the side of the convex thoracic scoliosis curve is always smaller in volume (mean difference 59 +/- 39 mi). The affected side also presents a smaller areola, a higher position of the nipple (mean difference 2.2 +/- 1.3 cm) and a higher position of the inframammary fold (mean difference 2.1 +/- 1.4 cm) when compared to the opposite breast. Though the asymmetry is predictable, the degree to which the patient presents these changes does not correlate with the severity of the scoliosis (Cobb angle). We believe that the severity of the asymmetry is a result of the difference between the hypoplastic breast and the normal breasts. In women with very large opposite breasts the asymmetry appears to be worse.

Evaluation of rib microstructure in Wistar rats using SR-µCT after radiation therapy simulation for breast cancer.

A better understanding of biological interactions that occur after exposure to photon radiation is needed in order to optimize therapeutic regimens and facilitate development and strategies that decrease radiation-induced side effects in humans. In this work, ribs of Wistar rat submitted to radiotherapy simulation were imaged using synchrotron radiation computed microtomography at Elettra Synchrotron Laboratory in Trieste, Italy. Histomorphometric parameters were calculated directly from the 3D microtomographic images and showed significant differences between irradiated and non-irradiated groups.

Condrosarcoma tóraco-abdominal / Thoraco-abdominal chondrosarcoma

Chondrosarcomas represent the third primary malignant bone tumor. Costal location occurs in 12 percent, being the most common tumor of the ribs. It affects most frequently in the second and fifth decades of life. This tumor is histologically classified into grades 1, 2 and 3 in descending order of differentiation. For grade 2, with oncologic resection, there is a 10-year survival of 64 percent, and a 10 percent risk for metastasis. Radiotherapy has a limited therapeutic role and chemotherapy has not shown benefits. We report a case of a 45 years old woman, who presented with a symptomatic growing mass in the right rib cage, of three months of evolution, whose evaluation by magnetic resonance imaging and computed tomography shows a sarcomatous tumor with involvement of ribs, muscles, diaphragm, pleura, peritoneum and liver. A small amount of intraperitoneal free fluid was observed. A block resection was made, resulting a 16.8 cm long at the widest point chondrosarcoma, grade 2, with involvement of all layers of the wall, tumor microfoci in the liver capsule and malignant tumor cells in peritoneal fluid.

Los condrosarcomas representan la tercera neoplasia ósea maligna primaria. La ubicación costal representa el 12 por ciento, siendo el tumor más frecuente de las costillas. Se presenta con mayor frecuencia en la segunda y quinta décadas de la vida. Este tumor se clasifica histológicamente en grados 1, 2 y 3 en orden decreciente de diferenciación. Para el grado 2, resecado oncológicamente, se observa una sobrevida a 10 años del 64 por ciento; existiendo un 10 por ciento de riesgo de metástasis. La radioterapia tiene un escaso rol terapéutico y la quimioterapia no ha demostrado beneficios. Presentamos el caso de una mujer de 45 años que consultó por un aumento de volumen sintomático de la parrilla costal derecha, de tres meses de evolución, cuya evaluación a través de tomografía axial y resonancia magnética permitió delinear un tumor sarcomatoso de la pared con afectación de costillas, músculos, diafragma, pleura, peritoneo e hígado. Además se observaba escasa cantidad de líquido libre intraperitoneal. Fue resecado en block un condrosarcoma de 16,8 cm de eje mayor, grado 2, con compromiso de todos los planos de la pared, microfocos tumorales en cápsula hepática y células neoplásicas malignas en líquido peritoneal.

Spondylocostal dysostosis associated with methylmalonic aciduria.

Spondylocostal dysostosis (SCD) is a genetic disorder characterized by vertebral segmentation and formation defects associated with changes of the ribs. Autosomal dominant and recessive modes of inheritance have been reported. Methylmalonic aciduria (MMA) is an inborn error of propionate or cobalamin metabolism. It is an autosomal recessive disorder and one of the most frequent forms of branched-chain organic acidurias. Here we report on a case of a Brazilian boy with both diseases. As we know, it is the first case in the literature with the occurrence of both SCD and MMA--the first a skeletal disease and the latter an inborn error of metabolism.